Wilms tumor is one of the most common malignant abdominal neoplasm in childhood, but Wilms tumor in adult is extremely rare. The tumor is characterized by a number of karyotypic and congenital associations, including deletons in the short arms of chromosome 1l, snd occasionally trisomy, hemihypertrophy, aniridia and urogenital abnormality. Histologically, the characteristic features are primitive or abortive glomeruli with apparent or poorly formed Bowman spaces, and abortive tubules enclosed within a spindle cell stroma. Optimal treatment of adult Wilms tumor is not established but generally followed that of childhood Wilms tumor include surgery, radiation and chemotherapy. Survival rate is influenced by stage and:hietologic pattern. We report a case of Wilms tumor in 23 year-old female.